38 research outputs found
ΠΠΏΡΡ ΠΏΡΠΈΠΌΠ΅Π½Π΅Π½ΠΈΡ Π°ΠΊΡΠΎΠ²Π΅Π³ΠΈΠ½Π° ΠΏΡΠΈ ΠΎΡΠ°Π³ΠΎΠ²ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ
ΠΠΎΠΌΠΏΠ»Π΅ΠΊΡΠ½Π°Ρ ΡΠ΅ΡΠ°ΠΏΠΈΡ ΡΠΎΡΡΠ΄ΠΈΡΡΡΡ Π½Π°ΡΡΡΠ΅Π½ΠΈΠΉ Ρ Π±ΠΎΠ»ΡΠ½ΡΡ ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠ΅ΠΉ
Scleroderma systematica (SDS) is a disease in which vascular diseases underlie the pathogenesis and presented by diverse clinical manifestations. Raynaud's syndrome and digital ulceration are the most common symptom of the diseases, which influences the quality of life in patients and requires continuous drug therapy. The paper discusses the recent European guidelines for the management of this manifestation of SDS. The proposed recommendations cannot unfortunately be realized in full measure now due to the unavailability of some drugs. The authors give their clinical experience with therapy for the vascular manifestations of SDS.Π‘ΠΈΡΡΠ΅ΠΌΠ½Π°Ρ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΡ (Π‘Π‘Π) - Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠ΅, ΠΏΡΠΈ ΠΊΠΎΡΠΎΡΠΎΠΌ ΡΠΎΡΡΠ΄ΠΈΡΡΡΠ΅ Π½Π°ΡΡΡΠ΅Π½ΠΈΡ Π»Π΅ΠΆΠ°Ρ Π² ΠΎΡΠ½ΠΎΠ²Π΅ ΠΏΠ°ΡΠΎΠ³Π΅Π½Π΅Π·Π° ΠΈ ΠΏΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½Ρ ΡΠ°Π·Π½ΠΎΠΎΠ±ΡΠ°Π·Π½ΡΠΌΠΈ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΠΌΠΈ ΠΏΡΠΎΡΠ²Π»Π΅Π½ΠΈΡΠΌΠΈ. Π‘ΠΈΠ½Π΄ΡΠΎΠΌ Π Π΅ΠΉΠ½ΠΎ ΠΈ ΠΎΠ±ΡΠ°Π·ΠΎΠ²Π°Π½ΠΈΠ΅ Π΄ΠΈΠ³ΠΈΡΠ°Π»ΡΠ½ΡΡ
ΡΠ·Π² - Π½Π°ΠΈΠ±ΠΎΠ»Π΅Π΅ ΡΠ°ΡΡΡΠΉ ΡΠΈΠΌΠΏΡΠΎΠΌ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡ, Π²Π»ΠΈΡΡΡΠΈΠΉ Π½Π° ΠΊΠ°ΡΠ΅ΡΡΠ²ΠΎ ΠΆΠΈΠ·Π½ΠΈ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² ΠΈ ΡΡΠ΅Π±ΡΡΡΠΈΠΉ ΠΏΠΎΡΡΠΎΡΠ½Π½ΠΎΠΉ Π»Π΅ΠΊΠ°ΡΡΡΠ²Π΅Π½Π½ΠΎΠΉ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ. Π ΡΡΠ°ΡΡΠ΅ ΠΎΠ±ΡΡΠΆΠ΄Π°ΡΡΡΡ Π½Π΅Π΄Π°Π²Π½ΠΎ ΠΎΠΏΡΠ±Π»ΠΈΠΊΠΎΠ²Π°Π½Π½ΡΠ΅ Π΅Π²ΡΠΎΠΏΠ΅ΠΉΡΠΊΠΈΠ΅ ΡΠ΅ΠΊΠΎΠΌΠ΅Π½Π΄Π°ΡΠΈΠΈ ΠΏΠΎ Π»Π΅ΡΠ΅Π½ΠΈΡ ΡΡΠΎΠ³ΠΎ ΠΏΡΠΎΡΠ²Π»Π΅Π½ΠΈΡ Π‘Π‘Π. ΠΡΠΏΠΎΠ»Π½Π΅Π½ΠΈΠ΅ ΠΏΡΠ΅Π΄Π»ΠΎΠΆΠ΅Π½Π½ΡΡ
ΡΠ΅ΠΊΠΎΠΌΠ΅Π½Π΄Π°ΡΠΈΠΉ Π² ΠΏΠΎΠ»Π½ΠΎΠΌ ΠΎΠ±ΡΠ΅ΠΌΠ΅, ΠΊ ΡΠΎΠΆΠ°Π»Π΅Π½ΠΈΡ, Π² Π½Π°ΡΡΠΎΡΡΠ΅Π΅ Π²ΡΠ΅ΠΌΡ Π½Π΅Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎ ΠΈΠ·-Π·Π° Π½Π΅Π΄ΠΎΡΡΡΠΏΠ½ΠΎΡΡΠΈ Π½Π΅ΠΊΠΎΡΠΎΡΡΡ
ΠΏΡΠ΅ΠΏΠ°ΡΠ°ΡΠΎΠ². ΠΠ²ΡΠΎΡΠ°ΠΌΠΈ ΠΏΡΠΈΠ²ΠΎΠ΄ΠΈΡΡΡ ΡΠΎΠ±ΡΡΠ²Π΅Π½Π½ΡΠΉ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈΠΉ ΠΎΠΏΡΡ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ ΡΠΎΡΡΠ΄ΠΈΡΡΡΡ
ΠΏΡΠΎΡΠ²Π»Π΅Π½ΠΈΠΉ Π‘Π‘Π
SCLERODERMA SYSTEMATICA WITH INTERSTITIAL LUNG LESION: COMPARATIVE CLINICAL CHARACTERISTICSWITH PATIENTS WITHOUT LUNG LESION
Objective. To compare disease history data and clinical and laboratory parameters in patients with scleroderma systematica (SDS) with high-resolution computed tomography (HRCT)-verified interstitial lung lesion (ILL) versus those without lung involvement. Subjects and methods. An examination was made in 138 patients with SDS who had been consecutively admitted in 2006-2008, female/male ratio, 124 : 14; limited : diffuse : mixed forms, 78 : 40 : 20; mean age, 47Β±13 years; median disease duration, 6 (2.5 11) years. The history data (occupational hazards, smoking, respiratory diseases) and clinical manifestations of SDS and laboratory data were studied. The diagnosis of ILL was established on the basis of chest HRCT. Results. According to HRCT data, the signs of varying ILL were found in 82% of the patients with SDS. The duration of SDS was similar in the patients with and without lung involvement; but the latter were younger at the time of disease onset. There were no significant differences between the groups compared in history data, clinical forms of SDS, the frequency of involvement of visceral organs and systems. Crepitation was heard only in the patients with ILL. The frequency of respiratory manifestations increased with a larger number of the involved lung segments. The prevalence of ILL was found to be positively correlated with age at the onset of SDS (r=0.29;
Π€ΠΈΠ±ΡΠΎΠ·ΠΈΡΡΡΡΠ°Ρ Π°ΡΡΡΠΎΠΏΠ°ΡΠΈΡ ΠΏΡΠΈ ΡΠ²Π΅Π½ΠΈΠ»ΡΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ
The group of scleroderma diseases includes a number of clinical entities, the main symptom of which is skin tightening. Scleroderma is a prominent example of these diseases, characterized by excessive synthesis and deposition of collagen in organs and tissues. A patient with juvenile systemic scleroderma with induration of the skin and underlying tissues, and persistent contractures of large joints since childhood, is described. This clinical example illustrates disease course peculiarities and differential diagnosis of systemic and limited (focal) scleroderma and scleroderma-like conditions in pediatric patients. The feasibility of pathogenetic therapy aimed at improving patient's the quality of life with formed disease phenotype is shown.Π Π³ΡΡΠΏΠΏΠ΅ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΡΠ΅ΡΠΊΠΈΡ
Π±ΠΎΠ»Π΅Π·Π½Π΅ΠΉ ΠΎΡΠ½ΠΎΡΠΈΡΡΡ ΡΡΠ΄ Π½ΠΎΠ·ΠΎΠ»ΠΎΠ³ΠΈΠΉ, ΠΎΡΠ½ΠΎΠ²Π½ΡΠΌ ΠΏΡΠΈΠ·Π½Π°ΠΊΠΎΠΌ ΠΊΠΎΡΠΎΡΡΡ
ΡΠ²Π»ΡΠ΅ΡΡΡ ΡΠΏΠ»ΠΎΡΠ½Π΅Π½ΠΈΠ΅ ΠΊΠΎΠΆΠΈ. Π‘ΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΡ β ΡΡΠΊΠΈΠΉ ΠΏΡΠ΅Π΄ΡΡΠ°Π²ΠΈΡΠ΅Π»Ρ ΡΡΠΈΡ
Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΠΉ, Ρ
Π°ΡΠ°ΠΊΡΠ΅ΡΠΈΠ·ΡΡΡΠΈΡ
ΡΡ ΠΈΠ·Π±ΡΡΠΎΡΠ½ΡΠΌ ΡΠΈΠ½ΡΠ΅Π·ΠΎΠΌ ΠΈ ΠΎΡΠ»ΠΎΠΆΠ΅Π½ΠΈΠ΅ΠΌ ΠΊΠΎΠ»Π»Π°Π³Π΅Π½Π° Π² ΠΎΡΠ³Π°Π½Π°Ρ
ΠΈ ΡΠΊΠ°Π½ΡΡ
. ΠΠΏΠΈΡΠ°Π½Π° Π±ΠΎΠ»ΡΠ½Π°Ρ ΡΠ²Π΅Π½ΠΈΠ»ΡΠ½ΠΎΠΉ ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠ΅ΠΉ Ρ ΠΈΠ½Π΄ΡΡΠ°ΡΠΈΠ΅ΠΉ ΠΊΠΎΠΆΠΈ ΠΈ ΠΏΠΎΠ΄Π»Π΅ΠΆΠ°ΡΠΈΡ
ΡΠΊΠ°Π½Π΅ΠΉ, Π° ΡΠ°ΠΊΠΆΠ΅ ΡΡΠΎΠΉΠΊΠΈΠΌΠΈ ΠΊΠΎΠ½ΡΡΠ°ΠΊΡΡΡΠ°ΠΌΠΈ ΠΊΡΡΠΏΠ½ΡΡ
ΡΡΡΡΠ°Π²ΠΎΠ² Ρ Π΄Π΅ΡΡΠΊΠΎΠ³ΠΎ Π²ΠΎΠ·ΡΠ°ΡΡΠ°. ΠΠ° ΡΡΠΎΠΌ ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΎΠΌ ΠΏΡΠΈΠΌΠ΅ΡΠ΅ ΡΠ°ΡΡΠΌΠ°ΡΡΠΈΠ²Π°ΡΡΡΡ ΠΎΡΠΎΠ±Π΅Π½Π½ΠΎΡΡΠΈ ΡΠ΅ΡΠ΅Π½ΠΈΡ ΠΈ Π΄ΠΈΡΡΠ΅ΡΠ΅Π½ΡΠΈΠ°Π»ΡΠ½Π°Ρ Π΄ΠΈΠ°Π³Π½ΠΎΡΡΠΈΠΊΠ° ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΠΈ ΠΎΠ³ΡΠ°Π½ΠΈΡΠ΅Π½Π½ΠΎΠΉ (ΠΎΡΠ°Π³ΠΎΠ²ΠΎΠΉ) ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ ΠΈ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΎΠΏΠΎΠ΄ΠΎΠ±Π½ΡΡ
ΡΠΎΡΡΠΎΡΠ½ΠΈΠΉ Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Π΄Π΅ΡΡΠΊΠΎΠ³ΠΎ Π²ΠΎΠ·ΡΠ°ΡΡΠ°. ΠΠΎΠΊΠ°Π·Π°Π½Ρ Π²ΠΎΠ·ΠΌΠΎΠΆΠ½ΠΎΡΡΠΈ ΠΏΠΎΠ΄Π±ΠΎΡΠ° ΠΏΠ°ΡΠΎΠ³Π΅Π½Π΅ΡΠΈΡΠ΅ΡΠΊΠΎΠΉ ΡΠ΅ΡΠ°ΠΏΠΈΠΈ, Π½Π°ΠΏΡΠ°Π²Π»Π΅Π½Π½ΠΎΠΉ Π½Π° ΡΠ»ΡΡΡΠ΅Π½ΠΈΠ΅ ΠΊΠ°ΡΠ΅ΡΡΠ²Π° ΠΆΠΈΠ·Π½ΠΈ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ² Ρ ΡΠΆΠ΅ ΡΡΠΎΡΠΌΠΈΡΠΎΠ²Π°Π½Π½ΡΠΌ ΡΠ΅Π½ΠΎΡΠΈΠΏΠΎΠΌ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡ
STUDY OF THE EFFICIENCY AND SAFETY OF MYCOPHENOLATE MOFETIL THERAPY IN PATIENTSWITH SYSTEMIC SCLERODERMA
Interstitial lung disease (ILD) is one of the major causes of death in systemic scleroderma (SSD). Treatment of these patients remains difficult and controversial. Mycophenolate mofetil (MPM) has been in vitro shown to inhibit overproduction of type I collagen and hence may be effective against SSD. Objective: to study the efficiency and safety of MPM therapy in patients with SSD and clinically relevant ILD in an open-label prospective study. Subjects and methods. Ten patients with SSD (7 and 3 with its diffuse and limited forms, respectively) and ILD were given MPM in combination with glucocorticoids (mean daily dose was 10+4 mg). The mean MPM therapy duration was 11.4+1.3 months. The Rodnan total skin thickness score, flexion index, forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide (DLCO), and European Scleroderma Study Group (EScSG) activity index were estimated and a 6-minute walk test (6MWT) was carried out before and after MPM therapy. Results. After therapy, the whole group showed a significant reduction in skin scores from 12.9+9.8 to 5.6+3.2 (p=0.036) and EScSG from 3.9+1.4 to 2.25+1.03 (p=0.015) and an increase in exercise tolerance from 446+155 to 535+78 m (p=0.03) as evidenced by 6MWT. The degree of flexion contractures decreased from 15+21 to 3.7+11.3 mm (p>0.05). FVC (77.8+18.7% versus 73.8+11.3%) and DLCO (45+14.4% versus 42+16.4%) were significantly unchanged. A 10% or more clinically significant fall was noted in FVC and DLCO in 3 and 1 patients, respectively. In the remaining patients, the lung functional test results remained stable. MPM tolerability was satisfactory. All the patients completed their course of treatment. Conclusion. Stabilization of lung function with higher exercise tolerance and significantly reduced skin density allow therapy with MPM in combination with low-dose glucocorticoids to be regarded as an effective and well-tolerated treatment in patients with ILD in the presence of SS
Comparative analysis of anxiety-depressive spectrum disorders in patients with rheumatic diseases
Research objective - comparative analysis of incidence and structure of anxiety-depressive spectrum disorders (ADD) in patients with various rheumatic diseases (RD). Materials and methods. 613 patients with RD were enrolled in the study: 180 with a reliable diagnosis of systemic lupus erythematosus (SLE), 128 with rheumatoid arthritis (RA), 110 with systemic sclerosis (SSc), 115 with Behcet's disease (BD), 80 with primary SjΓΆgren's syndrome (pSS). Female prevailed in all groups (95% of patients with pSS, 88,2% - SSc, 87,2% - RA, 85,5% of SLE) except BD patients (70% male). The mean age was 42.3Β±1.54 years and was lower in patients with BD (33.3Β±0.98 years) and SLE (34.6Β±0.93 years) compared to patients with SSc (49.9Β±2.47 years), RA (47.4Β±0.99 years) and pSS (46.2Β±2.3 years). The mean RD duration was 130,0Β±8,65 months and was more at BD - 148,5Β±10,4 months, pSS - 141,6Β±8,92 months, RA - 138,4Β±10,1months, and less at SLE - 134,9Β±8,8 months and SSc - 87,0Β±5,04 months. The mean SLE activity index SLEDAI was 9,13Β±0,63 points (high), RA (DAS28) - 5,26Β±0,17 points (high), BD (BDCAF) - 3,79Β±0,2 points (moderate) and SSc by G. Valentini - 1,1Β±0,20 points (moderate). Glucocorticoids took 100% of patients with pSS, 91,1% - SLE, 90% - SSc, 87% - BD and 67,2% - RA patients; conventional disease modifying anti-rheumatic drugs (cDMARDs) took 90% of patients with SSc, 84% - BD, 79,6% - RA, 68% - pSS, 40,6% - SLE. Biologic DMARDs took 32% of patients with RA, 17,4% - BD, 7,3% - SSc and 7,2% - SLE. Mental disorders were diagnosed by psychiatrist as a result of screening by the hospital anxiety and depression scale (HADS) and in semi-structured interview in accordance with the ICD-10/ DSM-IV. The severity of depression was evaluated by Montgomery-Asberg Depression Rating Scale (MADRS) and anxiety - by Hamilton Anxiety Rating Scale (HAM-A). Projective psychological methods were used for cognitive impairment detection. Results. Screening of depressive disorders (HADS-Dβ₯8) was positive in 180 (29,4%) patients with RD, including 74 (41%) patients with SLE, 38 (35%) - SSc, 29 (23%) - RA, 23 (20%) - BD and 16 (20%) - pSS; anxiety disorders (HADS-Aβ₯8) - in 272 (44,4%) patients, including 66 (52%) patients with RA, 40 (50%) - pSS, 77 (43%) - SLE, 45 (41%) - SSc and 44 (38%) - BD. In accordance with the ICD-10/ DSM-IV depressive disorders have been identified in 389 (63%) patients, including 94 (73%) patients with RA, 71 (64,5%) - SSc, 69 (60%) - BD, 90 (50%) - SLE and 39 (49%) - pSS; anxiety disorders - in 377 (61,5%) patients, including 20 (25%) patients with pSS, 44 (24,5%) - SLE, 29 (23%) - RA, 20 (17%) - BD and 7 (6,4%) - SSc. Conclusion. Anxiety-depressive spectrum disorders are typical for most patients with RA, SLE, SSc, pSS and BD. ADDs diagnosis in RD patients with the use of the HADS did not reveal a significant proportion. To obtain objective data on the frequency and structure of ADDs, psychopathological and clinical psychological diagnosis is necessary
ΠΡΠΏΠΎΠ»ΡΠ·ΠΎΠ²Π°Π½ΠΈΠ΅ ΠΌΠ΅ΠΆΠ΄ΡΠ½Π°ΡΠΎΠ΄Π½ΠΎΠ³ΠΎ ΠΈΠ½Π΄Π΅ΠΊΡΠ° Π΄Π»Ρ ΠΎΡΠ΅Π½ΠΊΠΈ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΠΈ ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ
Up to now, it is difficult to determine systemic scleroderma (SSD) activity because of the lack of validated tools to estimate changes in the pathological process. Attempts have been made to develop unified activity assessing methods for many years. The indices proposed by the European SSD Group are most popular today. This paper gives the results of using this index in a cohort of Russian patients.ΠΠΏΡΠ΅Π΄Π΅Π»Π΅Π½ΠΈΠ΅ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΠΈ ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ (Π‘Π‘Π) Π΄ΠΎ Π½Π°ΡΡΠΎΡΡΠ΅Π³ΠΎ Π²ΡΠ΅ΠΌΠ΅Π½ΠΈ Π·Π°ΡΡΡΠ΄Π½Π΅Π½ΠΎ ΠΈΠ·-Π·Π° ΠΎΡΡΡΡΡΡΠ²ΠΈΡ Π²Π°Π»ΠΈΠ΄ΠΈΡΠΎΠ²Π°Π½Π½ΡΡ
ΠΈΠ½ΡΡΡΡΠΌΠ΅Π½ΡΠΎΠ² Π΄Π»Ρ ΠΎΡΠ΅Π½ΠΊΠΈ ΠΈΠ·ΠΌΠ΅Π½Π΅Π½ΠΈΠΉ ΠΏΠ°ΡΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ ΠΏΡΠΎΡΠ΅ΡΡΠ°. Π ΡΠ΅ΡΠ΅Π½ΠΈΠ΅ ΠΌΠ½ΠΎΠ³ΠΈΡ
Π»Π΅Ρ ΠΏΡΠ΅Π΄ΠΏΡΠΈΠ½ΠΈΠΌΠ°ΡΡΡΡ ΠΏΠΎΠΏΡΡΠΊΠΈ ΡΠΎΠ·Π΄Π°Π½ΠΈΡ ΡΠ½ΠΈΡΠΈΡΠΈΡΠΎΠ²Π°Π½Π½ΡΡ
ΠΌΠ΅ΡΠΎΠ΄ΠΎΠ² ΠΎΡΠ΅Π½ΠΊΠΈ Π°ΠΊΡΠΈΠ²Π½ΠΎΡΡΠΈ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡ. ΠΠ°ΠΈΠ±ΠΎΠ»Π΅Π΅ ΠΏΠΎΠΏΡΠ»ΡΡΠ½ΡΠΌΠΈ Π½Π° ΡΠ΅Π³ΠΎΠ΄Π½ΡΡΠ½ΠΈΠΉ Π΄Π΅Π½Ρ ΡΠ²Π»ΡΡΡΡΡ ΠΈΠ½Π΄Π΅ΠΊΡΡ, ΠΏΡΠ΅Π΄Π»ΠΎΠΆΠ΅Π½Π½ΡΠ΅ Π΅Π²ΡΠΎΠΏΠ΅ΠΉΡΠΊΠΎΠΉ Π³ΡΡΠΏΠΏΠΎΠΉ ΠΏΠΎ ΠΈΠ·ΡΡΠ΅Π½ΠΈΡ Π‘Π‘Π. Π ΡΡΠ°ΡΡΠ΅ ΠΏΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½Ρ ΡΠ΅Π·ΡΠ»ΡΡΠ°ΡΡ ΠΈΡΠΏΠΎΠ»ΡΠ·ΠΎΠ²Π°Π½ΠΈΡ ΡΡΠΎΠ³ΠΎ ΠΈΠ½Π΄Π΅ΠΊΡΠ° Ρ ΠΊΠΎΠ³ΠΎΡΡΡ ΡΠΎΡΡΠΈΠΉΡΠΊΠΈΡ
ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ²
ΠΠ·ΠΎΠ»ΠΈΡΠΎΠ²Π°Π½Π½ΠΎΠ΅ ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΠ΅ Π΄ΠΈΡΡΡΠ·ΠΈΠΎΠ½Π½ΠΎΠΉ ΡΠΏΠΎΡΠΎΠ±Π½ΠΎΡΡΠΈ Π»Π΅Π³ΠΊΠΈΡ ΠΏΡΠΈ ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ Π±Π΅Π· Π»Π΅Π³ΠΎΡΠ½ΠΎΠΉ Π°ΡΡΠ΅ΡΠΈΠ°Π»ΡΠ½ΠΎΠΉ Π³ΠΈΠΏΠ΅ΡΡΠ΅Π½Π·ΠΈΠΈ: Π΄Π»ΠΈΡΠ΅Π»ΡΠ½ΠΎΠ΅ ΠΏΡΠΎΡΠΏΠ΅ΠΊΡΠΈΠ²Π½ΠΎΠ΅ Π½Π°Π±Π»ΡΠ΄Π΅Π½ΠΈΠ΅
The objective of this 5year prospective study was to investigate a clinical role of isolated decrease of DLCO in patients with systemic sclerosis (SS)Β without pulmonary arterial hypertension (PAH).Methods. We selected 48 out of 142 patients with SS: 35 (73%) with limited SS and 13 (27%) withΒ diffuse SS. The average length of the disease was 12.9 Β± 7.9 years. Inclusion criteria were DLCO < 80% pred., forced vital capacity (FVC) β₯ 80%Β pred. and systolic pulmonary artery pressure (PAP) β€ 35 mm Hg according to echocardiographic examination (echoCG). High resolution computed tomography (HRCT), spirometry, DLCO measurement, and echoCG were obtained at baseline and after 4.7 Β± 1 year of follow up. All patientsΒ were treated with standard therapy.Results. CT signs of interstitial lung disease (ILD) were found in 43 (89.6%) patients at baseline and newly developed in 3 other patients during the followup. During the followup, lung CT improved in 5 (10.4%) patients and progressed in 15 (31.3%) patients.Β Over 5 years, FVC did not change significantly (97.6 Β± 10.7% and 100.8 Β± 18.9%; Ρ = 0.15), while DLCO significantly decreased both in limitedΒ and diffuse SS groups (59.8 Β± 13.5% and 56.3 Β± 12%; Ρ = 0.006). Mean PAP values remained within normal range in majority of patients. ClinicallyΒ significant FVC reduction (β₯ 10%) was found in 5 patients; of them, CT signs of ILD at baseline were seen in 3 patients and newly developed during the followup in 2 others. Clinically significant DLCO reduction (β₯ 10%) was documented in 11 (23%) patients, all had CT signs of ILD at baseline, although CT progression during the followup was noted only in six of them. Contemporary deterioration in FVC, DLCO and CT was found in 3 patients.Conclusion. Clinical course of ILD in SS patients with isolated DLCO reduction and without PAH was relatively benign, with respiratoryΒ volumes being preserved within normal range for long time. Comparison of radiological and functional changes in a prospective study has suggested that DLCO is a more sensitive tool to determine ILD progression compared to HRCT. Regular DLCO measurements could be used as a reliable toolΒ for monitoring of ILD associated with SS.ΠΠ½ΡΠ΅ΡΡΡΠΈΡΠΈΠ°Π»ΡΠ½ΠΎΠ΅ ΠΏΠΎΡΠ°ΠΆΠ΅Π½ΠΈΠ΅ Π»Π΅Π³ΠΊΠΈΡ
(ΠΠΠ) ΠΏΡΠΈ ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ (Π‘Π‘Π), ΠΊΠ°ΠΊ ΠΏΡΠ°Π²ΠΈΠ»ΠΎ, ΡΠΎΠΏΡΠΎΠ²ΠΎΠΆΠ΄Π°Π΅ΡΡΡ ΡΠ΅ΡΡΡΠΈΠΊΡΠΈΠ²Π½ΡΠΌΠΈΒ Π½Π°ΡΡΡΠ΅Π½ΠΈΡΠΌΠΈ Π»Π΅Π³ΠΎΡΠ½ΠΎΠΉ ΡΡΠ½ΠΊΡΠΈΠΈ, ΠΏΡΠΈ ΡΡΠΎΠΌ ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΠ΅ Π΄ΡΡ
Π°ΡΠ΅Π»ΡΠ½ΡΡ
ΠΎΠ±ΡΠ΅ΠΌΠΎΠ² ΠΈ Π΄ΠΈΡΡΡΠ·ΠΈΠΎΠ½Π½ΠΎΠΉ ΡΠΏΠΎΡΠΎΠ±Π½ΠΎΡΡΠΈ Π»Π΅Π³ΠΊΠΈΡ
(DLCO) ΠΏΡΠΎΠΈΡΡ
ΠΎΠ΄ΠΈΡΒ ΠΏΠ°ΡΠ°Π»Π»Π΅Π»ΡΠ½ΠΎ. Π ΡΡΠ΄Π΅ ΡΠ»ΡΡΠ°Π΅Π² ΠΏΡΠΈ ΠΠΠ Π²ΡΡΠ²Π»ΡΠ΅ΡΡΡ ΠΈΠ·ΠΎΠ»ΠΈΡΠΎΠ²Π°Π½Π½ΠΎΠ΅ ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΠ΅ DLCO, ΠΊΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΎΠ΅ Π·Π½Π°ΡΠ΅Π½ΠΈΠ΅ ΠΊΠΎΡΠΎΡΠΎΠ³ΠΎ ΠΎΡΡΠ°Π΅ΡΡΡ Π΄ΠΎ ΠΊΠΎΠ½ΡΠ°Β Π½Π΅ΡΡΠ½ΡΠΌ.Π¦Π΅Π»Ρ. ΠΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠ°Ρ ΠΎΡΠ΅Π½ΠΊΠ° ΠΈΠ·ΠΎΠ»ΠΈΡΠΎΠ²Π°Π½Π½ΠΎΠ³ΠΎ ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΡ DLCO Ρ Π±ΠΎΠ»ΡΠ½ΡΡ
Π‘Π‘Π Π±Π΅Π· Π»Π΅Π³ΠΎΡΠ½ΠΎΠΉ Π°ΡΡΠ΅ΡΠΈΠ°Π»ΡΠ½ΠΎΠΉ Π³ΠΈΠΏΠ΅ΡΡΠ΅Π½Π·ΠΈΠΈ (ΠΠΠ)Β Π² 5Π»Π΅ΡΠ½Π΅ΠΌ ΠΏΡΠΎΡΠΏΠ΅ΠΊΡΠΈΠ²Π½ΠΎΠΌ ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΈ.ΠΠ°ΡΠ΅ΡΠΈΠ°Π»Ρ ΠΈ ΠΌΠ΅ΡΠΎΠ΄Ρ. ΠΠ· Π±ΠΎΠ»ΡΠ½ΡΡ
(n = 142), Π½Π°Π±Π»ΡΠ΄Π°Π²ΡΠΈΡ
ΡΡ Π² Π€Π΅Π΄Π΅ΡΠ°Π»ΡΠ½ΠΎΠΌ Π³ΠΎΡΡΠ΄Π°ΡΡΡΠ²Π΅Π½Π½ΠΎΠΌΒ Π±ΡΠ΄ΠΆΠ΅ΡΠ½ΠΎΠΌ Π½Π°ΡΡΠ½ΠΎΠΌ ΡΡΡΠ΅ΠΆΠ΄Π΅Π½ΠΈΠΈ Β«ΠΠ°ΡΡΠ½ΠΎΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°ΡΠ΅Π»ΡΡΠΊΠΈΠΉ ΠΈΠ½ΡΡΠΈΡΡΡ ΡΠ΅Π²ΠΌΠ°ΡΠΎΠ»ΠΎΠ³ΠΈΠΈ ΠΈΠΌΠ΅Π½ΠΈ Π.Π.ΠΠ°ΡΠΎΠ½ΠΎΠ²ΠΎΠΉΒ», ΠΎΡΠΎΠ±ΡΠ°Π½Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΡΒ (n = 48) Ρ Π»ΠΈΠΌΠΈΡΠΈΡΠΎΠ²Π°Π½Π½ΠΎΠΉ β 35 (73 %) ΠΈ Π΄ΠΈΡΡΡΠ·Π½ΠΎΠΉ β 13 (27 %) ΡΠΎΡΠΌΠ°ΠΌΠΈ Π‘Π‘Π (Π΄Π°Π²Π½ΠΎΡΡΡ Π‘Π‘Π β 12,9 Β± 7,9 Π³ΠΎΠ΄Π°; ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΠ΅ DLCO < 80 %;Β ΡΠΎΡΡΠΈΡΠΎΠ²Π°Π½Π½Π°Ρ ΠΆΠΈΠ·Π½Π΅Π½Π½Π°Ρ Π΅ΠΌΠΊΠΎΡΡΡ Π»Π΅Π³ΠΊΠΈΡ
(Π€ΠΠΠ) β₯ 80 %Π΄ΠΎΠ»ΠΆ., ΠΎΡΡΡΡΡΡΠ²ΠΈΠ΅ ΠΠΠ ΠΏΠΎ Π΄Π°Π½Π½ΡΠΌ ΡΡ
ΠΎΠΊΠ°ΡΠ΄ΠΈΠΎΠ³ΡΠ°ΡΠΈΠΈ (ΠΡ
ΠΎΠΠ); ΡΠΈΡΡΠΎΠ»ΠΈΡΠ΅ΡΠΊΠΎΠ΅Β Π΄Π°Π²Π»Π΅Π½ΠΈΠ΅ Π² Π»Π΅Π³ΠΎΡΠ½ΠΎΠΉ Π°ΡΡΠ΅ΡΠΈΠΈ (Π‘ΠΠΠ) β€ 35 ΠΌΠΌ ΡΡ. ΡΡ.). ΠΠΎΠΌΠΏΡΡΡΠ΅ΡΠ½Π°Ρ ΡΠΎΠΌΠΎΠ³ΡΠ°ΡΠΈΡ Π²ΡΡΠΎΠΊΠΎΠ³ΠΎ ΡΠ°Π·ΡΠ΅ΡΠ΅Π½ΠΈΡ (ΠΠ’ΠΠ ), ΡΠΏΠΈΡΠΎΠΌΠ΅ΡΡΠΈΡ,Β ΠΎΠΏΡΠ΅Π΄Π΅Π»Π΅Π½ΠΈΠ΅ DLCO, ΠΡ
ΠΎΠΠ ΠΏΡΠΎΠ²ΠΎΠ΄ΠΈΠ»ΠΈΡΡ ΠΏΡΠΈ Π²ΠΊΠ»ΡΡΠ΅Π½ΠΈΠΈ Π² ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠ΅ ΠΈ ΡΠ΅ΡΠ΅Π· 4,7 Β± 1,0 Π³ΠΎΠ΄Π°. ΠΡΠ΅ ΠΏΠ°ΡΠΈΠ΅Π½ΡΡ ΠΏΠΎΠ»ΡΡΠ°Π»ΠΈ ΡΡΠ°Π½Π΄Π°ΡΡΠ½ΡΡΒ ΡΠ΅ΡΠ°ΠΏΠΈΡ.Π Π΅Π·ΡΠ»ΡΡΠ°ΡΡ. ΠΡΠΈ Π²ΠΊΠ»ΡΡΠ΅Π½ΠΈΠΈ Π² ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠ΅ ΠΠ’ΠΏΡΠΈΠ·Π½Π°ΠΊΠΈ ΠΠΠ ΠΎΠ±Π½Π°ΡΡΠΆΠ΅Π½Ρ Ρ 43 (89,6 %) Π±ΠΎΠ»ΡΠ½ΡΡ
ΠΈ ΡΠ΅ΡΠ΅Π· 5 Π»Π΅Ρ ΠΏΠΎΡΠ²ΠΈΠ»ΠΈΡΡ Π΅ΡΠ΅Β Ρ 3; ΠΏΡΠΈ ΡΡΠΎΠΌ ΠΏΠΎΠ»ΠΎΠΆΠΈΡΠ΅Π»ΡΠ½Π°Ρ ΠΠ’Π΄ΠΈΠ½Π°ΠΌΠΈΠΊΠ° ΠΎΡΠΌΠ΅ΡΠ΅Π½Π° Ρ 5 (10,4 %), ΠΏΡΠΎΠ³ΡΠ΅ΡΡΠΈΡΠΎΠ²Π°Π½ΠΈΠ΅ β Ρ 15 (31,3 %) ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ². ΠΠΎΠΊΠ°Π·Π°ΡΠ΅Π»ΠΈ Π€ΠΠΠΒ Π² ΡΡΠ΅Π΄Π½Π΅ΠΌ ΠΏΠΎ Π³ΡΡΠΏΠΏΠ΅ Π·Π½Π°ΡΠΈΠΌΠΎ Π½Π΅ ΠΈΠ·ΠΌΠ΅Π½ΠΈΠ»ΠΈΡΡ (97,6 Β± 10,7 ΠΈ 100,8 Β± 18,9 ΡΠΎΠΎΡΠ²Π΅ΡΡΡΠ²Π΅Π½Π½ΠΎ; Ρ = 0,15), DLCO Π΄ΠΎΡΡΠΎΠ²Π΅ΡΠ½ΠΎ ΡΠ½ΠΈΠ·ΠΈΠ»Π°ΡΡ (59,8 Β±Β 13,5 ΠΈ 56,3 Β± 12,0 % ΡΠΎΠΎΡΠ²Π΅ΡΡΡΠ²Π΅Π½Π½ΠΎ; Ρ = 0,006). ΠΠΎΠΊΠ°Π·Π°ΡΠ΅Π»ΠΈ Π‘ΠΠΠ ΠΎΡΡΠ°Π²Π°Π»ΠΈΡΡ Π² ΠΏΡΠ΅Π΄Π΅Π»Π°Ρ
Π½ΠΎΡΠΌΠ°Π»ΡΠ½ΡΡ
Π·Π½Π°ΡΠ΅Π½ΠΈΠΉ Ρ Π±ΠΎΠ»ΡΡΠΈΠ½ΡΡΠ²Π°Β ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ². ΠΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈ Π·Π½Π°ΡΠΈΠΌΠΎΠ΅ (β₯ 10 %) ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΠ΅ Π€ΠΠΠ Π²ΡΡΠ²Π»Π΅Π½ΠΎ Ρ 5 ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ², ΠΈΠ· Π½ΠΈΡ
Π² 3 ΡΠ»ΡΡΠ°ΡΡ
ΠΠ’ΠΏΡΠΈΠ·Π½Π°ΠΊΠΈ ΠΠΠ ΠΎΡΠΌΠ΅ΡΠ΅Π½ΡΒ ΠΏΡΠΈ Π²ΠΊΠ»ΡΡΠ΅Π½ΠΈΠΈ Π² ΠΈΡΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠ΅, Π² 2 β ΠΏΠΎΡΠ²ΠΈΠ»ΠΈΡΡ Π² Π΄ΠΈΠ½Π°ΠΌΠΈΠΊΠ΅. ΠΠ»ΠΈΠ½ΠΈΡΠ΅ΡΠΊΠΈ Π·Π½Π°ΡΠΈΠΌΠΎΠ΅ ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΠ΅ DLCO (β₯ 10 %) ΠΎΠ±Π½Π°ΡΡΠΆΠ΅Π½ΠΎ Ρ 11 (23 %)Β ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΎΠ². Π£ Π²ΡΠ΅Ρ
ΠΏΡΠΈ 1ΠΌ ΠΎΠ±ΡΠ»Π΅Π΄ΠΎΠ²Π°Π½ΠΈΠΈ Π²ΡΡΠ²Π»Π΅Π½Ρ ΠΠ’ΠΏΡΠΈΠ·Π½Π°ΠΊΠΈ ΠΠΠ, ΠΎΠ΄Π½Π°ΠΊΠΎ ΡΠΎΠ»ΡΠΊΠΎ Π² 6 ΡΠ»ΡΡΠ°ΡΡ
Π² Π΄ΠΈΠ½Π°ΠΌΠΈΠΊΠ΅ ΠΎΡΠΌΠ΅ΡΠ°Π»ΠΎΡΡ Π½Π°ΡΠ°ΡΡΠ°Π½ΠΈΠ΅Β ΡΠ΅Π½ΡΠ³Π΅Π½ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΡ
ΠΏΡΠΈΠ·Π½Π°ΠΊΠΎΠ² ΠΠΠ. ΠΠ°ΡΠ°Π»Π»Π΅Π»ΡΠ½ΠΎΠ΅ ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΠ΅ Π€ΠΠΠ ΠΈ DLCO, ΡΠΎΠΏΡΠΎΠ²ΠΎΠΆΠ΄Π°Π²ΡΠ΅Π΅ΡΡ ΠΏΡΠΎΠ³ΡΠ΅ΡΡΠΈΡΠΎΠ²Π°Π½ΠΈΠ΅ΠΌ ΡΠ΅Π½ΡΠ³Π΅Π½ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΈΡ
ΠΏΡΠΈΠ·Π½Π°ΠΊΠΎΠ² ΠΠΠ, Π²ΡΡΠ²Π»Π΅Π½ΠΎ Ρ 3 Π±ΠΎΠ»ΡΠ½ΡΡ
.ΠΠ°ΠΊΠ»ΡΡΠ΅Π½ΠΈΠ΅. Π’Π΅ΡΠ΅Π½ΠΈΠ΅ ΠΠΠ Ρ Π±ΠΎΠ»ΡΠ½ΡΡ
Π‘Π‘Π Ρ ΠΈΠ·ΠΎΠ»ΠΈΡΠΎΠ²Π°Π½Π½ΡΠΌ ΡΠ½ΠΈΠΆΠ΅Π½ΠΈΠ΅ΠΌ DLCO Π±Π΅Π·Β ΠΠΠ Π±ΡΠ»ΠΎ ΠΎΡΠ½ΠΎΡΠΈΡΠ΅Π»ΡΠ½ΠΎ Π΄ΠΎΠ±ΡΠΎΠΊΠ°ΡΠ΅ΡΡΠ²Π΅Π½Π½ΡΠΌ Ρ ΡΠΎΡ
ΡΠ°Π½Π΅Π½ΠΈΠ΅ΠΌ Π»Π΅Π³ΠΎΡΠ½ΡΡ
ΠΎΠ±ΡΠ΅ΠΌΠΎΠ² Π² ΠΏΡΠ΅Π΄Π΅Π»Π°Ρ
Π½ΠΎΡΠΌΠ°Π»ΡΠ½ΡΡ
Π·Π½Π°ΡΠ΅Π½ΠΈΠΉ Π² ΡΠ΅ΡΠ΅Π½ΠΈΠ΅ Π΄Π»ΠΈΡΠ΅Π»ΡΠ½ΠΎΠ³ΠΎΒ Π²ΡΠ΅ΠΌΠ΅Π½ΠΈ. ΠΡΠΈ ΡΠΎΠΏΠΎΡΡΠ°Π²Π»Π΅Π½ΠΈΠΈ ΡΠ΅Π½ΡΠ³Π΅Π½ΠΎΠ»ΠΎΠ³ΠΈΡΠ΅ΡΠΊΠΎΠΉ Π΄ΠΈΠ½Π°ΠΌΠΈΠΊΠΈ ΠΈ Π»Π΅Π³ΠΎΡΠ½ΡΡ
ΡΠ΅ΡΡΠΎΠ² Π² ΠΏΡΠΎΡΠΏΠ΅ΠΊΡΠΈΠ²Π½ΠΎΠΌ Π½Π°Π±Π»ΡΠ΄Π΅Π½ΠΈΠΈ ΠΎΡΠΌΠ΅ΡΠ΅Π½ΠΎ, ΡΡΠΎ Π² ΠΎΡΡΠ°ΠΆΠ΅Π½ΠΈΠΈ ΠΏΡΠΎΠ³ΡΠ΅ΡΡΠΈΡΠΎΠ²Π°Π½ΠΈΡ ΠΠΠ DLCO ΡΠ²Π»ΡΠ΅ΡΡΡ Π±ΠΎΠ»Π΅Π΅ ΡΡΠ²ΡΡΠ²ΠΈΡΠ΅Π»ΡΠ½ΡΠΌ ΡΠ΅ΡΡΠΎΠΌ, ΡΠ΅ΠΌ ΠΠ’ΠΠ . Π Π΅Π³ΡΠ»ΡΡΠ½ΠΎΠ΅ ΠΎΠΏΡΠ΅Π΄Π΅Π»Π΅Π½ΠΈΠ΅ DLCO ΠΌΠΎΠΆΠ΅Ρ Π±ΡΡΡΒ ΠΈΠ½ΡΠΎΡΠΌΠ°ΡΠΈΠ²Π½ΡΠΌ ΠΈΠ½ΡΡΡΡΠΌΠ΅Π½ΡΠΎΠΌ Π΄ΠΈΠ½Π°ΠΌΠΈΡΠ΅ΡΠΊΠΎΠ³ΠΎ Π½Π°Π±Π»ΡΠ΄Π΅Π½ΠΈΡ Π±ΠΎΠ»ΡΠ½ΡΡ
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